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https://www.readbyqxmd.com/read/29764427/col4a5-and-lama5-variants-co-inherited-in-familial-hematuria-digenic-inheritance-or-genetic-modifier-effect
#1
Konstantinos Voskarides, Gregory Papagregoriou, Despina Hadjipanagi, Ioanelli Petrou, Isavella Savva, Avraam Elia, Yiannis Athanasiou, Androulla Pastelli, Maria Kkolou, Michalis Hadjigavriel, Christoforos Stavrou, Alkis Pierides, Constantinos Deltas
BACKGROUND: About 40-50% of patients with familial microscopic hematuria (FMH) caused by thin basement membrane nephropathy (TBMN) inherit heterozygous mutations in collagen IV genes (COL4A3, COL4A4). On long follow-up, the full phenotypic spectrum of these patients varies a lot, ranging from isolated MH or MH plus low-grade proteinuria to chronic renal failure of variable degree, including end-stage renal disease (ESRD). METHODS: Here, we performed Whole Exome Sequencing (WES) in patients of six families, presenting with autosomal dominant FMH, with or without progression to proteinuria and loss of renal function, all previously found negative for severe collagen IV mutations...
May 16, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29760984/the-value-of-ultrahigh-resolution-oct-in-dermatology-delineating-the-dermo-epidermal-junction-capillaries-in-the-dermal-papillae-and-vellus-hairs
#2
Niels Møller Israelsen, Michael Maria, Mette Mogensen, Sophie Bojesen, Mikkel Jensen, Merete Haedersdal, Adrian Podoleanu, Ole Bang
Optical coherence tomography (OCT) imaging of the skin is gaining recognition and is increasingly applied to dermatological research. A key dermatological parameter inferred from an OCT image is the epidermal (Ep) thickness as a thickened Ep can be an indicator of a skin disease. Agreement in the literature on the signal characters of Ep and the subjacent skin layer, the dermis (D), is evident. Ambiguities of the OCT signal interpretation in the literature is however seen for the transition region between the Ep and D, which from histology is known as the dermo-epidermal junction (DEJ); a distinct junction comprised of the lower surface of a single cell layer in epidermis (the stratum basale) connected to an even thinner membrane (the basement membrane)...
May 1, 2018: Biomedical Optics Express
https://www.readbyqxmd.com/read/29707110/characterization-of-mirna-and-their-target-gene-during-chicken-embryo-skeletal-muscle-development
#3
Endashaw Jebessa, Hongjia Ouyang, Bahareldin Ali Abdalla, Zhenhui Li, Auwalu Yusuf Abdullahi, Qingshen Liu, Qinghua Nie, Xiquan Zhang
MicroRNAs (miRNAs) are non-coding RNAs that regulate mRNA expression by degradation or translational inhibition. We investigated the underlying molecular mechanisms of skeletal muscle development based on differentially expressed genes and miRNAs. We compared mRNA and miRNA from chicken skeletal muscle at embryonic day E11, E16 and one day post-hatch (P1). The interaction networks were constructed, according to target prediction results and integration analysis of up-regulated genes with down regulated miRNAs or down-regulated genes with up-regulated miRNAs with |log2fold change| ≥ 1...
April 3, 2018: Oncotarget
https://www.readbyqxmd.com/read/29673759/alport-syndrome-and-pierson-syndrome-diseases-of-the-glomerular-basement-membrane
#4
REVIEW
Steven D Funk, Meei-Hua Lin, Jeffrey H Miner
The glomerular basement membrane (GBM) is an important component of the kidney's glomerular filtration barrier. Like all basement membranes, the GBM contains type IV collagen, laminin, nidogen, and heparan sulfate proteoglycan. It is flanked by the podocytes and glomerular endothelial cells that both synthesize it and adhere to it. Mutations that affect the GBM's collagen α3α4α5(IV) components cause Alport syndrome (kidney disease with variable ear and eye defects) and its variants, including thin basement membrane nephropathy...
April 16, 2018: Matrix Biology: Journal of the International Society for Matrix Biology
https://www.readbyqxmd.com/read/29669314/three-novel-heterozygous-col4a4-mutations-result-in-three-different-collagen-type-iv-kidney-disease-phenotypes
#5
Ang Li, Er-Zhi Gao, Ying-Xia Cui, Jian-Hong Liu, Xing Lv, Xiu-Xiu Wei, Xin-Yi Xia, Chun-Lin Gao, Feng-Xia Liu, Zheng-Kun Xia, Asan, Zhi-Hong Liu, Xiao-Jun Li
Thin basement membrane nephropathy (TBMN), autosomal dominant Alport syndrome (ADAS), and focal segmental glomerulosclerosis (FSGS) are kidney diseases that differ in clinical diagnosis, treatment, and prognosis. Nevertheless, they may result from the same causative genes. Here, we report 3 COL4A4 heterozygous mutations (p.Gly208Arg, p.Ser513Glufs*2, and p.Met1617Cysfs*39) that lead to 3 different collagen type IV kidney disease phenotypes, manifesting as TBMN, ADAS, and FSGS. Using bioinformatics analyses and pedigree verification, we show that these novel variants are pathogenetic and cosegregate with TBMN, ADAS, and FSGS...
2018: Cytogenetic and Genome Research
https://www.readbyqxmd.com/read/29597206/curcumin-alleviates-diabetic-retinopathy-in-experimental-diabetic-rats
#6
Fang Yang, Jinqiang Yu, Feng Ke, Mei Lan, Dekun Li, Ke Tan, Jiaojiao Ling, Ying Wang, Kaili Wu, Dai Li
PURPOSE: To investigate the potential protective effects of curcumin on the retina in diabetic rats. METHODS: An experimental diabetic rat model was induced by a low dose of streptozotocin combined with a high-energy diet. Rats which had blood glucose levels ≥11.6 mmol/L were used as diabetic rats. The diabetic rats were randomly divided into 3 groups: diabetic rats with no treatment (DM), diabetic rats treated with 100 mg/kg curcumin (DM + Cur 100 mg/kg), and diabetic rats treated with 200 mg/kg curcumin (DM + Cur 200 mg/kg)...
March 29, 2018: Ophthalmic Research
https://www.readbyqxmd.com/read/29530752/a-unique-evolution-of-the-kidney-phenotype-in-a-patient-with-autosomal-recessive-alport-syndrome
#7
Gisella Vischini, Meghan E Kapp, Ferrin C Wheeler, Laszlo Hopp, Agnes B Fogo
Alport syndrome is due to mutations in one of the genes encoding (α3,4,5) type IV collagen resulting in defective type IV collagen, a key component of the glomerular basement membrane (GBM). The GBM is initially thin, and with ongoing remodeling, develops a thickened basket-woven appearance. We report a unique case of a 9-year-old boy who was biopsied for hematuria and proteinuria, diagnosed as IgA nephropathy, with normal GBM appearance and thickness. Due to a family history of hematuria and chronic kidney disease, he subsequently underwent genetic evaluation and a mutation of α3 type IV collagen (COL4A3) was detected...
March 9, 2018: Human Pathology
https://www.readbyqxmd.com/read/29473159/developmental-and-degenerative-cardiac-defects-in-the-taiwanese-mouse-model-of-severe-spinal-muscular-atrophy
#8
Gillian K Maxwell, Eva Szunyogova, Hannah K Shorrock, Thomas H Gillingwater, Simon H Parson
Spinal muscular atrophy (SMA), an autosomal recessive disease caused by a decrease in levels of the survival motor neuron (SMN) protein, is the most common genetic cause of infant mortality. Although neuromuscular pathology is the most severe feature of SMA, other organs and tissues, including the heart, are also known to be affected in both patients and animal models. Here, we provide new insights into changes occurring in the heart, predominantly at pre- and early symptomatic ages, in the Taiwanese mouse model of severe SMA...
February 22, 2018: Journal of Anatomy
https://www.readbyqxmd.com/read/29334529/midterm-outcome-of-kidney-transplantation-from-donors-with-thin-basement-membrane-nephropathy
#9
Chanjoong Choi, Sanghyun Ahn, Seung-Kee Min, Jongwon Ha, Curie Ahn, Yonsu Kim, Hajeong Lee, Sang-Il Min
BACKGROUND: Thin basement membrane nephropathy (TBMN) is the most common cause of persistent glomerular hematuria. Most individuals with TBMN show a benign course, although it can be difficult to distinguish it from early stages of progressive renal diseases. However, only limited studies address the prognosis of donors with TBMN and their recipients. METHODS: From 2007 to 2016, 11 recipients received kidney grafts from donors with TBMN, and their clinical data were analyzed retrospectively...
April 2018: Transplantation
https://www.readbyqxmd.com/read/29246972/increased-capillary-tortuosity-and-pericapillary-basement-membrane-thinning-in-skeletal-muscle-of-mice-undergoing-running-wheel-training
#10
Oliver Baum, Carole Sollberger, Andrea Raaflaub, Adolfo Odriozola, Gunnar Spohr, Sebastian Frese, Stefan A Tschanz
To work out which microvascular remodeling processes occur in murine skeletal muscle during endurance exercise, we subjected C57BL/6 mice to voluntary running wheel training for 1 week (1 wk-t) or 6 weeks (6 wks-t). By means of morphometry, the capillarity as well as the compartmental and sub-compartmental structure of the capillaries were quantitatively described at the light microscopy level and at the electron microscopy level, respectively, in the plantaris (PLNT) muscle of the exercising mice in comparison to untrained littermates...
February 19, 2018: Journal of Experimental Biology
https://www.readbyqxmd.com/read/29230854/adjuvant-potential-of-virgin-coconut-oil-extract-on-antiretroviral-therapy-induced-testicular-toxicity-an-ultrastructural-study
#11
O O Ogedengbe, A I Jegede, I O Onanuga, U Offor, A I Peter, E N Akang, E C S Naidu, O O Azu
The effects of Virgin coconut oil as an adjuvant to highly active antiretroviral therapy (HAART) were investigated on the testicular ultrastructure and biochemical markers in rats. Twenty male Sprague-Dawley rats, weighing 153-169 g were divided into four groups and treated as follows: control A (distilled water), B (HAART), C (HAART+Virgin coconut oil 10 ml/kg) and D (Virgin coconut oil [VCO] 10 ml/kg). Testicular segments were evaluated using transmission electron microscopy. Serum was assayed for testosterone, luteinising hormone, follicle stimulating hormone and testicular tissue for malondialdehyde and glutathione...
April 2018: Andrologia
https://www.readbyqxmd.com/read/29198685/thin-glomerular-basement-membrane-in-a-kidney-transplant-of-an-alport-s-syndrome-patient-a-case-report
#12
S Santos, S Marques, T Golper, A Langone, A B Fogo
Alport syndrome (AS) and thin basement membrane lesions are caused by various mutations in type IV collagen genes. Although AS is considered a rare disease, thin basement membrane is a frequent pattern, especially in families with a history of persistent hematuria. We report a patient with a diagnosis of AS who developed end-stage kidney disease (ESKD) and received a kidney transplant from a living unrelated donor. The graft biopsy specimen surprisingly showed a pattern of thin basement membranes.
December 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/29172845/temporal-retinal-thinning-and-the-diagnosis-of-alport-syndrome-and-thin-basement-membrane-nephropathy
#13
Yan Chen, Deb Colville, Francesco Ierino, Andrew Symons, Judy Savige
BACKGROUND AND OBJECTIVES: Alport syndrome is an inherited disease characterized by renal failure, hearing loss, and ocular abnormalities, including temporal retinal thinning. This study compared retinal thinning in Alport syndrome and other renal diseases. METHODS: Alport syndrome was diagnosed on renal biopsy and genetic testing. Subjects underwent optical coherence tomography (OCT) (Spectralis OCT, Heidelberg Instruments). Retinal thinning was determined from horizontal macular OCT scans through the foveal center using the formula: Temporal thickness index (TTI) = (nasal - temporal thickness) ÷ nasal thickness × 100%, and compared with the normal range for each age group...
April 2018: Ophthalmic Genetics
https://www.readbyqxmd.com/read/29164232/mast-cell-derived-tryptase-in-geographic-atrophy
#14
D Scott McLeod, Imran Bhutto, Malia M Edwards, Manasee Gedam, Rajkumar Baldeosingh, Gerard A Lutty
Purpose: Our previous study demonstrated significantly more degranulating mast cells (MCs) in choroids from subjects with age-related macular degeneration compared to aged controls. This study examined the immunolocalization of tryptase, the most abundant MC secretory granule-derived serine protease, in aged control eyes and eyes with geographic atrophy (GA). Methods: Postmortem human eyes with and without GA were obtained from the National Disease Research Interchange...
November 1, 2017: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/29138824/genetic-mutational-testing-of-chinese-children-with-familial-hematuria-with-biopsy%C3%A2-proven-fsgs
#15
Yongzhen Li, Ying Wang, Qingnan He, Xiqiang Dang, Yan Cao, Xiaochuan Wu, Shuanghong Mo, Xiaoxie He, Zhuwen Yi
Focal segmental glomerulosclerosis (FSGS) is a pathological lesion rather than a disease, with a diverse etiology. FSGS may result from genetic and non‑genetic factors. FSGS is considered a podocyte disease due to the fact that in the majority of patients with proven‑FSGS, the lesion results from defects in the podocyte structure or function. However, FSGS does not result exclusively from podocyte‑associated genes, however also from other genes including collagen IV‑associated genes. Patients who carry the collagen type IVA3 chain (COL4A3) or COL4A4 mutations usually exhibit Alport Syndrome (AS), thin basement membrane neuropathy or familial hematuria (FH)...
January 2018: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29129537/a-moving-source-of-matrix-components-is-essential-for-de-novo-basement-membrane-formation
#16
Yutaka Matsubayashi, Adam Louani, Anca Dragu, Besaiz J Sánchez-Sánchez, Eduardo Serna-Morales, Lawrence Yolland, Attila Gyoergy, Gema Vizcay, Roland A Fleck, John M Heddleston, Teng-Leong Chew, Daria E Siekhaus, Brian M Stramer
The basement membrane (BM) is a thin layer of extracellular matrix (ECM) beneath nearly all epithelial cell types that is critical for cellular and tissue function. It is composed of numerous components conserved among all bilaterians [1]; however, it is unknown how all of these components are generated and subsequently constructed to form a fully mature BM in the living animal. Although BM formation is thought to simply involve a process of self-assembly [2], this concept suffers from a number of logistical issues when considering its construction in vivo...
November 20, 2017: Current Biology: CB
https://www.readbyqxmd.com/read/28923616/histopathologic-findings-of-potential-kidney-donors-with-asymptomatic-microscopic-hematuria-impact-on-donation
#17
E A Hassan, T Z Ali, A Abdulbaki, I A Ibrahim, H M Almanae, H A Aleid
INTRODUCTION: Isolated microscopic hematuria (IMH) is not uncommon in potential kidney donors. AIM: The aim was to study the kidney biopsy findings of potential kidney donors with IMH and the impact of the histopathologic diagnoses on the decision to accept or decline such donors from kidney donation. METHODS: In this retrospective study, all the potential kidney donors with IMH were identified from the medical records of patients who underwent kidney biopsies between January 2010 and December 2016...
October 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28905837/-en-face-optical-coherence-tomography-findings-in-a-case-of-alport-syndrome
#18
In Hwan Cho, Hoon Dong Kim, Sang Joon Jung, Tae Kwann Park
Alport syndrome is a rare hereditary disease that is associated with retinal abnormalities such as dot-and-fleck retinopathy and temporal macular thinning. The main pathophysiological process of Alport syndrome is loss of the collagen network in the basement membrane. However, the alterations in each retinal layer have not been fully evaluated. In the case presented here, we evaluated the retina of a patient with Alport syndrome using en face optical coherence tomography (OCT). The findings suggested that the primary alterations occur in the internal limiting membrane and the retinal pigment epithelium basement membrane which is a part of the Bruch's membrane...
September 2017: Indian Journal of Ophthalmology
https://www.readbyqxmd.com/read/28881511/-role-of-igg-antibody-to-galactose-deficient-iga1-in-children-with-iga-nephropathy
#19
N Zhou, H Zhang, X R Liu, Q Sun, Q Meng, Z Chen, Y Shen
Objective: In order to learn the serum level of galactose-deficient IgA1 (GdIgA1), IgG antibody to galactose-deficient IgA1(GdIgA1-IgG) and the clinical role of them in IgA nephropathy(IgAN) children. Method: We compared blood levels of GdIgA1, GdIgA1-IgG in 33 children with IgAN, 38 children with other renal disease (including focal segmental glomerular nephritis, minimal change disease, Alport syndrome and thin basement membrane nephropathy) as disease controls, 35 healthy children as normal controls with enzyme-linked immunosorbent assay(ELISA)...
September 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28830057/histopathological-and-ultrastructural-changes-experimentally-induced-by-bee-venom-in-seminiferous-epithelium-via-structural-functional-alteration-of-sertoli-cells
#20
Adrian Florea, Constantin Puică, Sami Hamed, Mariana Tilinca, Horea Matei
We tested here the ability of bee venom (BV) to interfere with spermatogenesis in rats in two experimental conditions. The histopathological changes were assessed with brightfield microscopy using a novel staining technique, based on methylene blue, orange G and ponceau xylidine. Transmission electron microscopy was also used to identify fine subcellular changes. BV injection for 30days in daily doses of 700μg BV/kg resulted in reducing testicular weight, along with significant larger diameters of seminiferous tubules and reduced number of Sertoli cells (SCs)...
November 2017: Micron: the International Research and Review Journal for Microscopy
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